-
1
-
-
5044239107
-
Pathways of chaperone-mediated protein folding in the cytosol
-
Young, J.C.; Agashe, V.R.; Siegers, K.; Hartl, F.U. Pathways of chaperone-mediated protein folding in the cytosol. Nat. Rev. Mol. Cell Biol., 2004, 5(10), 781-791.
-
(2004)
Nat. Rev. Mol. Cell Biol
, vol.5
, Issue.10
, pp. 781-791
-
-
Young, J.C.1
Agashe, V.R.2
Siegers, K.3
Hartl, F.U.4
-
2
-
-
38549119467
-
Chaperones in control of protein disaggregation
-
Liberek, K.; Lewandowska, A.; Zietkiewicz, S. Chaperones in control of protein disaggregation. EMBO J., 2008, 27(2), 328-335.
-
(2008)
EMBO J
, vol.27
, Issue.2
, pp. 328-335
-
-
Liberek, K.1
Lewandowska, A.2
Zietkiewicz, S.3
-
3
-
-
0021256895
-
Alzheimer's disease: Initial report of the purification and characterization of a novel cerebrovascular amyloid protein
-
Glenner, G.G.; Wong, C.W. Alzheimer's disease: initial report of the purification and characterization of a novel cerebrovascular amyloid protein. Biochem. Biophys. Res. Commun., 1984, 120(3), 885-890.
-
(1984)
Biochem. Biophys. Res. Commun
, vol.120
, Issue.3
, pp. 885-890
-
-
Glenner, G.G.1
Wong, C.W.2
-
4
-
-
0023009658
-
Microtubule-associated protein tau. A component of Alzheimer paired helical filaments
-
Grundke-Iqbal, I.; Iqbal, K.; Quinlan, M.; Tung, Y.C.; Zaidi, M.S.; Wisniewski, H.M. Microtubule-associated protein tau. A component of Alzheimer paired helical filaments. J. Biol. Chem., 1986, 261(13), 6084-6089.
-
(1986)
J. Biol. Chem
, vol.261
, Issue.13
, pp. 6084-6089
-
-
Grundke-Iqbal, I.1
Iqbal, K.2
Quinlan, M.3
Tung, Y.C.4
Zaidi, M.S.5
Wisniewski, H.M.6
-
5
-
-
0003986552
-
Isolation of a fragment of tau derived from the core of the paired helical filament of Alzheimer disease
-
Wischik, C.M.; Novak, M.; Thogersen, H.C.; Edwards, P.C.; Runswick, M.J.; Jakes, R.; Walker, J.E.; Milstein, C.; Roth, M.; Klug, A. Isolation of a fragment of tau derived from the core of the paired helical filament of Alzheimer disease. Proc. Natl. Acad. Sci. USA, 1988, 85(12), 4506-4510.
-
(1988)
Proc. Natl. Acad. Sci. USA
, vol.85
, Issue.12
, pp. 4506-4510
-
-
Wischik, C.M.1
Novak, M.2
Thogersen, H.C.3
Edwards, P.C.4
Runswick, M.J.5
Jakes, R.6
Walker, J.E.7
Milstein, C.8
Roth, M.9
Klug, A.10
-
6
-
-
0030882856
-
α-synuclein in Lewy bodies
-
Spillantini, M.G.; Schmidt, M.L.; Lee, V.M.; Trojanowski, J.Q.; Jakes, R.; Goedert, M. α-synuclein in Lewy bodies. Nature, 1997, 388(6645), 839-840.
-
(1997)
Nature
, vol.388
, Issue.6645
, pp. 839-840
-
-
Spillantini, M.G.1
Schmidt, M.L.2
Lee, V.M.3
Trojanowski, J.Q.4
Jakes, R.5
Goedert, M.6
-
7
-
-
0037077040
-
Toxic proteins in neurodegenerative disease
-
Taylor, J.P.; Hardy, J.; Fischbeck, K.H. Toxic proteins in neurodegenerative disease. Science, 2002, 296(5575), 1991-1995.
-
(2002)
Science
, vol.296
, Issue.5575
, pp. 1991-1995
-
-
Taylor, J.P.1
Hardy, J.2
Fischbeck, K.H.3
-
8
-
-
0037264120
-
Unfolding the role of protein misfolding in neurodegenerative diseases
-
Soto, C. Unfolding the role of protein misfolding in neurodegenerative diseases. Nat. Rev. Neurosci., 2003, 4(1), 49-60.
-
(2003)
Nat. Rev. Neurosci
, vol.4
, Issue.1
, pp. 49-60
-
-
Soto, C.1
-
9
-
-
27644596641
-
Opinion: What is the role of protein aggregation in neurodegeneration?
-
Ross, C.A.; Poirier, M.A. Opinion: What is the role of protein aggregation in neurodegeneration? Nat. Rev. Mol. Cell Biol., 2005, 6(11), 891-898.
-
(2005)
Nat. Rev. Mol. Cell Biol
, vol.6
, Issue.11
, pp. 891-898
-
-
Ross, C.A.1
Poirier, M.A.2
-
10
-
-
0026088977
-
Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease
-
Goate, A.; Chartier-Harlin, M.C.; Mullan, M.; Brown, J.; Crawford, F.; Fidani, L.; Giuffra, L.; Haynes, A.; Irving, N.; James, L.; Mant, R.; Newton, P.; Rooke, K.; Roques, P.; Talbot, C.; Pericak-Vance, M.; Roses, A.; Williamson, R.; Rossor, M.; Owen, M.; Hardy, J. Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease. Nature, 1991, 349(6311), 704-706.
-
(1991)
Nature
, vol.349
, Issue.6311
, pp. 704-706
-
-
Goate, A.1
Chartier-Harlin, M.C.2
Mullan, M.3
Brown, J.4
Crawford, F.5
Fidani, L.6
Giuffra, L.7
Haynes, A.8
Irving, N.9
James, L.10
Mant, R.11
Newton, P.12
Rooke, K.13
Roques, P.14
Talbot, C.15
Pericak-Vance, M.16
Roses, A.17
Williamson, R.18
Rossor, M.19
Owen, M.20
Hardy, J.21
more..
-
11
-
-
0029004341
-
-
Sherrington, R.; Rogaev, E.I.; Liang, Y.; Rogaeva, E.A.; Levesque, G.; Ikeda, M.; Chi, H.; Lin, C.; Li, G.; Holman, K.; Tsuda, T.; Mar, L.; Foncin, J.F.; Bruni, A.C.; Montesi, M.P.; Sorbi, S.; Rainero, I.; Pinessi, L.; Nee, L.; Chumakov, I.; Pollen, D.; Brookes, A.; Sanseau, P.; Polinsky, R.J.; Wasco, W.; Da Silva, H.A.R.; Haines, J.L.; Pericak-Vance, M.A.; Tanzi, R.E.; Roses, A.D.; Fraser, P.E.; Rommens, J.M.; St George-Hyslop, P.H. Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease. Nature, 1995, 375(6534), 754-760.
-
Sherrington, R.; Rogaev, E.I.; Liang, Y.; Rogaeva, E.A.; Levesque, G.; Ikeda, M.; Chi, H.; Lin, C.; Li, G.; Holman, K.; Tsuda, T.; Mar, L.; Foncin, J.F.; Bruni, A.C.; Montesi, M.P.; Sorbi, S.; Rainero, I.; Pinessi, L.; Nee, L.; Chumakov, I.; Pollen, D.; Brookes, A.; Sanseau, P.; Polinsky, R.J.; Wasco, W.; Da Silva, H.A.R.; Haines, J.L.; Pericak-Vance, M.A.; Tanzi, R.E.; Roses, A.D.; Fraser, P.E.; Rommens, J.M.; St George-Hyslop, P.H. Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease. Nature, 1995, 375(6534), 754-760.
-
-
-
-
12
-
-
0029087026
-
Candidate gene for the chromosome 1 familial Alzheimer's disease locus
-
Levy-Lahad, E.; Wasco, W.; Poorkaj, P.; Romano, D.M.; Oshima, J.; Pettingell, W.H.; Yu, C.E.; Jondro, P.D.; Schmidt, S.D.; Wang, K.; Crowley, A.C.; Fu, Y.H.; Guenette, S.Y.; Galas, D.; Nemens, E.; Wijsman, E.M.; Bird, T.D.; Schellenberg, G.D.; Tanzi, R.E. Candidate gene for the chromosome 1 familial Alzheimer's disease locus. Science, 1995, 269(5226), 973-977.
-
(1995)
Science
, vol.269
, Issue.5226
, pp. 973-977
-
-
Levy-Lahad, E.1
Wasco, W.2
Poorkaj, P.3
Romano, D.M.4
Oshima, J.5
Pettingell, W.H.6
Yu, C.E.7
Jondro, P.D.8
Schmidt, S.D.9
Wang, K.10
Crowley, A.C.11
Fu, Y.H.12
Guenette, S.Y.13
Galas, D.14
Nemens, E.15
Wijsman, E.M.16
Bird, T.D.17
Schellenberg, G.D.18
Tanzi, R.E.19
-
13
-
-
0029101491
-
Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene
-
Rogaev, E.I.; Sherrington, R.; Rogaeva, E.A.; Levesque, G.; Ikeda, M.; Liang, Y.; Chi, H.; Lin, C.; Holman, K.; Tsuda, T.; Mar, L.; Sorbi, S.; Nacmias, B.; Piacentini, S.; Amaducci, L.; Chumakov, I.; Cohen, D.; Lannfelt, L.; Fraser, P.E.; Rommens, J.M.; St George-Hyslop, P.H. Familial Alzheimer's disease in kindreds with missense mutations in a gene on chromosome 1 related to the Alzheimer's disease type 3 gene. Nature, 1995, 376(6543), 775-778.
-
(1995)
Nature
, vol.376
, Issue.6543
, pp. 775-778
-
-
Rogaev, E.I.1
Sherrington, R.2
Rogaeva, E.A.3
Levesque, G.4
Ikeda, M.5
Liang, Y.6
Chi, H.7
Lin, C.8
Holman, K.9
Tsuda, T.10
Mar, L.11
Sorbi, S.12
Nacmias, B.13
Piacentini, S.14
Amaducci, L.15
Chumakov, I.16
Cohen, D.17
Lannfelt, L.18
Fraser, P.E.19
Rommens, J.M.20
St George-Hyslop, P.H.21
more..
-
14
-
-
0037135111
-
The amyloid hypothesis of Alzheimer's disease: Progress and problems on the road to therapeutics
-
Hardy, J.; Selkoe, D.J. The amyloid hypothesis of Alzheimer's disease: progress and problems on the road to therapeutics. Science, 2002, 297(5580), 353-356.
-
(2002)
Science
, vol.297
, Issue.5580
, pp. 353-356
-
-
Hardy, J.1
Selkoe, D.J.2
-
15
-
-
0030744876
-
Mutation in the α-synuclein gene identified in families with Parkinson's disease
-
Polymeropoulos, M.H.; Lavedan, C.; Leroy, E.; Ide, S.E.; Dehejia, A.; Dutra, A.; Pike, B.; Root, H.; Rubenstein, J.; Boyer, R.; Stenroos, E.S.; Chandrasekharappa, S.; Athanassiadou, A.; Papapetropoulos, T.; Johnson, W.G.; Lazzarini, A.M.; Duvoisin, R.C.; Di Iorio, G.; Golbe, L.I.; Nussbaum, R.L. Mutation in the α-synuclein gene identified in families with Parkinson's disease. Science, 1997, 276(5321), 2045-2047.
-
(1997)
Science
, vol.276
, Issue.5321
, pp. 2045-2047
-
-
Polymeropoulos, M.H.1
Lavedan, C.2
Leroy, E.3
Ide, S.E.4
Dehejia, A.5
Dutra, A.6
Pike, B.7
Root, H.8
Rubenstein, J.9
Boyer, R.10
Stenroos, E.S.11
Chandrasekharappa, S.12
Athanassiadou, A.13
Papapetropoulos, T.14
Johnson, W.G.15
Lazzarini, A.M.16
Duvoisin, R.C.17
Di Iorio, G.18
Golbe, L.I.19
Nussbaum, R.L.20
more..
-
16
-
-
0242300619
-
α-Synuclein locus triplication causes Parkinson's disease
-
Singleton, A.B.; Farrer, M.; Johnson, J.; Singleton, A.; Hague, S.; Kachergus, J.; Hulihan, M.; Peuralinna, T.; Dutra, A.; Nussbaum, R.; Lincoln, S.; Crawley, A.; Hanson, M.; Maraganore, D.; Adler, C.; Cookson, M.R.; Muenter, M.; Baptista, M.; Miller, D.; Blancato, J.; Hardy, J.; Gwinn-Hardy, K. α-Synuclein locus triplication causes Parkinson's disease. Science, 2003, 302(5646), 841.
-
(2003)
Science
, vol.302
, Issue.5646
, pp. 841
-
-
Singleton, A.B.1
Farrer, M.2
Johnson, J.3
Singleton, A.4
Hague, S.5
Kachergus, J.6
Hulihan, M.7
Peuralinna, T.8
Dutra, A.9
Nussbaum, R.10
Lincoln, S.11
Crawley, A.12
Hanson, M.13
Maraganore, D.14
Adler, C.15
Cookson, M.R.16
Muenter, M.17
Baptista, M.18
Miller, D.19
Blancato, J.20
Hardy, J.21
Gwinn-Hardy, K.22
more..
-
17
-
-
68649095418
-
Molecular mechanisms of α-synuclein neurodegeneration
-
Waxman, E.A.; Giasson, B.I. Molecular mechanisms of α-synuclein neurodegeneration. Biochim. Biophys. Acta., 2009, 1792(7), 616-624.
-
(2009)
Biochim. Biophys. Acta
, vol.1792
, Issue.7
, pp. 616-624
-
-
Waxman, E.A.1
Giasson, B.I.2
-
18
-
-
0027401203
-
-
Rosen, D.R.; Siddique, T.; Patterson, D.; Figlewicz, D.A.; Sapp, P.; Hentati, A.; Donaldson, D.; Goto, J.; O'Regan, J.P.; Deng, H.X.; Rahmani, Z.: Krizus, A.; McKenna-Yasek, D.; Cayabyab, A.; Gaston, S.M.; Berger, R.; Tanzi, R.E.; Halperin, J.J.; Herzfeldt, B.; Van den Bergh, R.; Hung, W.; Bird, T.; Deng, G.; Mulder, D.W.; Smyth, C.; Laing, N.G.; Soriano, E.; Pericak-Vance, M.A.; Haines, J.; Rouleau, G.A.; Gusella, J.S.; Horvitz, H.R.; Brown, R.H. Jr. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 1993, 362(6415), 59-62.
-
Rosen, D.R.; Siddique, T.; Patterson, D.; Figlewicz, D.A.; Sapp, P.; Hentati, A.; Donaldson, D.; Goto, J.; O'Regan, J.P.; Deng, H.X.; Rahmani, Z.: Krizus, A.; McKenna-Yasek, D.; Cayabyab, A.; Gaston, S.M.; Berger, R.; Tanzi, R.E.; Halperin, J.J.; Herzfeldt, B.; Van den Bergh, R.; Hung, W.; Bird, T.; Deng, G.; Mulder, D.W.; Smyth, C.; Laing, N.G.; Soriano, E.; Pericak-Vance, M.A.; Haines, J.; Rouleau, G.A.; Gusella, J.S.; Horvitz, H.R.; Brown, R.H. Jr. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 1993, 362(6415), 59-62.
-
-
-
-
19
-
-
41149180753
-
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
-
Sreedharan, J.; Blair, I.P.; Tripathi, V.B.; Hu, X.; Vance, C.; Rogelj, B.; Ackerley, S.; Durnall, J.C.; Williams, K.L.; Buratti, E.; Baralle, F.; de Belleroche, J.; Mitchell, J.D.; Leigh, P.N.; Al-Chalabi, A.; Miller, C.C.; Nicholson, G.; Shaw, C.E. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science, 2008, 319(5870), 1668-1672.
-
(2008)
Science
, vol.319
, Issue.5870
, pp. 1668-1672
-
-
Sreedharan, J.1
Blair, I.P.2
Tripathi, V.B.3
Hu, X.4
Vance, C.5
Rogelj, B.6
Ackerley, S.7
Durnall, J.C.8
Williams, K.L.9
Buratti, E.10
Baralle, F.11
de Belleroche, J.12
Mitchell, J.D.13
Leigh, P.N.14
Al-Chalabi, A.15
Miller, C.C.16
Nicholson, G.17
Shaw, C.E.18
-
20
-
-
0032543684
-
-
Hutton, M, Lendon, C.L, Rizzu, P, Baker, M, Froelich, S, Houlden, H, Pickering-Brown, S, Chakraverty, S, Isaacs, A, Grover, A, Hackett, J, Adamson, J, Lincoln, S, Dickson, D, Davies, P, Petersen, R.C, Stevens, M, de Graaff, E, Wauters, E, van Baren, J, Hillebrand, M, Joosse, M, Kwon, J.M, Nowotny, P, Che, L.K, Norton, J, Morris, J.C, Reed, L.A, Trojanowski, J, Basun, H, Lannfelt, L, Neystat, M, Fahn, S, Dark, F, Tannenberg, T, Dodd, P.R, Hayward, N, Kwok, J.B, Schofield, P.R, Andreadis, A, Snowden, J, Craufurd, D, Neary, D, Owen, F, Oostra, B.A, Hardy, J, Goate, A, van Swieten, J, Mann, D, Lynch, T, Heutink, P. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature, 1998, 3936686, 702-705
-
Hutton, M.; Lendon, C.L.; Rizzu, P.; Baker, M.; Froelich, S.; Houlden, H.; Pickering-Brown, S.; Chakraverty, S.; Isaacs, A.; Grover, A.; Hackett, J.; Adamson, J.; Lincoln, S.; Dickson, D.; Davies, P.; Petersen, R.C.; Stevens, M.; de Graaff, E.; Wauters, E.; van Baren, J.; Hillebrand, M.; Joosse, M.; Kwon, J.M.; Nowotny, P.; Che, L.K.; Norton, J.; Morris, J.C.; Reed, L.A.; Trojanowski, J.; Basun, H.; Lannfelt, L.; Neystat, M.; Fahn, S.; Dark, F.; Tannenberg, T.; Dodd, P.R.; Hayward, N.; Kwok, J.B.; Schofield, P.R.; Andreadis, A.; Snowden, J.; Craufurd, D.; Neary, D.; Owen, F.; Oostra, B.A.; Hardy, J.; Goate, A.; van Swieten, J.; Mann, D.; Lynch, T.; Heutink, P. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature, 1998, 393(6686), 702-705.
-
-
-
-
21
-
-
0032560487
-
Mutation in the tau gene in familial multiple system tauopathy with presenile dementia
-
Spillantini, M.G.; Murrell, J.R.; Goedert, M.; Farlow, M.R.; Klug, A.; Ghetti, B. Mutation in the tau gene in familial multiple system tauopathy with presenile dementia. Proc. Natl. Acad. Sci. USA, 1998, 95(13), 7737-7741.
-
(1998)
Proc. Natl. Acad. Sci. USA
, vol.95
, Issue.13
, pp. 7737-7741
-
-
Spillantini, M.G.1
Murrell, J.R.2
Goedert, M.3
Farlow, M.R.4
Klug, A.5
Ghetti, B.6
-
22
-
-
14444284106
-
Tau is a candidate gene for chromosome 17 frontotemporal dementia
-
Poorkaj, P.; Bird, T.D.; Wijsman, E.; Nemens, E.; Garruto, R.M.; Anderson, L.; Andreadis, A.; Wiederholt, W.C.; Raskind, M.; Schellenberg, G.D. Tau is a candidate gene for chromosome 17 frontotemporal dementia. Ann. Neurol., 1998, 43(6), 815-825.
-
(1998)
Ann. Neurol
, vol.43
, Issue.6
, pp. 815-825
-
-
Poorkaj, P.1
Bird, T.D.2
Wijsman, E.3
Nemens, E.4
Garruto, R.M.5
Anderson, L.6
Andreadis, A.7
Wiederholt, W.C.8
Raskind, M.9
Schellenberg, G.D.10
-
23
-
-
33746919083
-
-
Baker, M.; Mackenzie, I.R.; Pickering-Brown, S.M.; Gass, J.; Rademakers, R.; Lindholm, C.; Snowden, J.; Adamson, J.; Sadovnick, A.D.; Rollinson, S.; Cannon, A.; Dwosh, E.; Neary, D.; Melquist, S.; Richardson, A.; Dickson, D.; Berger, Z.; Eriksen, J.; Robinson, T.; Zehr, C.; Dickey, C.A.; Crook, R.; McGowan, E.; Mann, D.; Boeve, B.; Feldman, H.; Hutton, M. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature, 2006, 442(7105), 916-919.
-
Baker, M.; Mackenzie, I.R.; Pickering-Brown, S.M.; Gass, J.; Rademakers, R.; Lindholm, C.; Snowden, J.; Adamson, J.; Sadovnick, A.D.; Rollinson, S.; Cannon, A.; Dwosh, E.; Neary, D.; Melquist, S.; Richardson, A.; Dickson, D.; Berger, Z.; Eriksen, J.; Robinson, T.; Zehr, C.; Dickey, C.A.; Crook, R.; McGowan, E.; Mann, D.; Boeve, B.; Feldman, H.; Hutton, M. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature, 2006, 442(7105), 916-919.
-
-
-
-
24
-
-
33746910649
-
Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21
-
Cruts, M.; Gijselinck, I.; van der Zee, J.; Engelborghs, S.; Wils, H.; Pirici, D.; Rademakers, R.; Vandenberghe, R.; Dermaut, B.; Martin, J.J.; van Duijn, C.; Peeters, K.; Sciot, R.; Santens, P.; De Pooter, T.; Mattheijssens, M.; Van den Broeck, M.; Cuijt, I.; Vennekens, K.; De Deyn, P.P.; Kumar-Singh, S.; Van Broeckhoven, C. Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21. Nature, 2006, 442(7105), 920-924.
-
(2006)
Nature
, vol.442
, Issue.7105
, pp. 920-924
-
-
Cruts, M.1
Gijselinck, I.2
van der Zee, J.3
Engelborghs, S.4
Wils, H.5
Pirici, D.6
Rademakers, R.7
Vandenberghe, R.8
Dermaut, B.9
Martin, J.J.10
van Duijn, C.11
Peeters, K.12
Sciot, R.13
Santens, P.14
De Pooter, T.15
Mattheijssens, M.16
Van den Broeck, M.17
Cuijt, I.18
Vennekens, K.19
De Deyn, P.P.20
Kumar-Singh, S.21
Van Broeckhoven, C.22
more..
-
25
-
-
34547692622
-
Trinucleotide repeat disorders
-
Orr, H.T.; Zoghbi, H.Y. Trinucleotide repeat disorders. Annu. Rev. Neurosci., 2007, 30, 575-621.
-
(2007)
Annu. Rev. Neurosci
, vol.30
, pp. 575-621
-
-
Orr, H.T.1
Zoghbi, H.Y.2
-
26
-
-
58949102251
-
Conformational changes and aggregation of expanded polyglutamine proteins as therapeutic targets of the polyglutamine diseases: Exposed β-sheet hypothesis
-
Nagai, Y.; Popiel, H.A. Conformational changes and aggregation of expanded polyglutamine proteins as therapeutic targets of the polyglutamine diseases: exposed β-sheet hypothesis. Curr. Pharm. Des., 2008, 14(30), 3267-3279.
-
(2008)
Curr. Pharm. Des
, vol.14
, Issue.30
, pp. 3267-3279
-
-
Nagai, Y.1
Popiel, H.A.2
-
27
-
-
69949170793
-
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
-
Bauer, P.O.; Nukina, N. The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies. J. Neurochem., 2009, 110(6), 1737-1765.
-
(2009)
J. Neurochem
, vol.110
, Issue.6
, pp. 1737-1765
-
-
Bauer, P.O.1
Nukina, N.2
-
28
-
-
0027480960
-
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
-
The Huntington's Disease Collaborative Research Group
-
The Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell, 1993, 72(6), 971-983.
-
(1993)
Cell
, vol.72
, Issue.6
, pp. 971-983
-
-
-
29
-
-
0027164698
-
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
-
Orr, H.T.; Chung, M.Y.; Banfi, S.; Kwiatkowski, T.J., Jr.; Servadio, A.; Beaudet, A.L.; McCall, A.E.; Duvick, L.A.; Ranum, L.P.; Zoghbi, H.Y. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nat. Genet., 1993, 4(3), 221-226.
-
(1993)
Nat. Genet
, vol.4
, Issue.3
, pp. 221-226
-
-
Orr, H.T.1
Chung, M.Y.2
Banfi, S.3
Kwiatkowski Jr., T.J.4
Servadio, A.5
Beaudet, A.L.6
McCall, A.E.7
Duvick, L.A.8
Ranum, L.P.9
Zoghbi, H.Y.10
-
30
-
-
0030292488
-
Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2
-
Pulst, S.M.; Nechiporuk, A.; Nechiporuk, T.; Gispert, S.; Chen, X.N.; Lopes-Cendes, I.; Pearlman, S.; Starkman, S.; Orozco-Diaz, G.; Lunkes, A.; DeJong, P.; Rouleau, G.A.; Auburger, G.; Korenberg, J.R.; Figueroa, C.; Sahba, S. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat. Genet., 1996, 14(3), 269-276.
-
(1996)
Nat. Genet
, vol.14
, Issue.3
, pp. 269-276
-
-
Pulst, S.M.1
Nechiporuk, A.2
Nechiporuk, T.3
Gispert, S.4
Chen, X.N.5
Lopes-Cendes, I.6
Pearlman, S.7
Starkman, S.8
Orozco-Diaz, G.9
Lunkes, A.10
DeJong, P.11
Rouleau, G.A.12
Auburger, G.13
Korenberg, J.R.14
Figueroa, C.15
Sahba, S.16
-
31
-
-
0030292368
-
Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT
-
Sanpei, K.; Takano, H.; Igarashi, S.; Sato, T.; Oyake, M.; Sasaki, H.; Wakisaka, A.; Tashiro, K.; Ishida, Y.; Ikeuchi, T.; Koide, R.; Saito, M.; Sato, A.; Tanaka, T.; Hanyu, S.; Takiyama, Y.; Nishizawa, M.; Shimizu, N.; Nomura, Y.; Segawa, M.; Iwabuchi, K.; Eguchi, I.; Tanaka, H.; Takahashi, H.; Tsuji, S. Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nat. Genet., 1996, 14(3), 277-284.
-
(1996)
Nat. Genet
, vol.14
, Issue.3
, pp. 277-284
-
-
Sanpei, K.1
Takano, H.2
Igarashi, S.3
Sato, T.4
Oyake, M.5
Sasaki, H.6
Wakisaka, A.7
Tashiro, K.8
Ishida, Y.9
Ikeuchi, T.10
Koide, R.11
Saito, M.12
Sato, A.13
Tanaka, T.14
Hanyu, S.15
Takiyama, Y.16
Nishizawa, M.17
Shimizu, N.18
Nomura, Y.19
Segawa, M.20
Iwabuchi, K.21
Eguchi, I.22
Tanaka, H.23
Takahashi, H.24
Tsuji, S.25
more..
-
32
-
-
0030294345
-
Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats
-
Imbert, G.; Saudou, F.; Yvert, G.; Devys, D.; Trottier, Y.; Garnier, J.M.; Weber, C.; Mandel, J.L.; Cancel, G.; Abbas, N.; Durr, A.; Didierjean, O.; Stevanin, G.; Agid, Y.; Brice, A. Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nat. Genet., 1996, 14(3), 285-291.
-
(1996)
Nat. Genet
, vol.14
, Issue.3
, pp. 285-291
-
-
Imbert, G.1
Saudou, F.2
Yvert, G.3
Devys, D.4
Trottier, Y.5
Garnier, J.M.6
Weber, C.7
Mandel, J.L.8
Cancel, G.9
Abbas, N.10
Durr, A.11
Didierjean, O.12
Stevanin, G.13
Agid, Y.14
Brice, A.15
-
33
-
-
0028143527
-
CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1
-
Kawaguchi, Y.; Okamoto, T.; Taniwaki, M.; Aizawa, M.; Inoue, M.; Katayama, S.; Kawakami, H.; Nakamura, S.; Nishimura, M.; Akiguchi, I.; Kimura, J.; Narumiya, S.; Kakizuka, A. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet., 1994, 8(3), 221-228.
-
(1994)
Nat. Genet
, vol.8
, Issue.3
, pp. 221-228
-
-
Kawaguchi, Y.1
Okamoto, T.2
Taniwaki, M.3
Aizawa, M.4
Inoue, M.5
Katayama, S.6
Kawakami, H.7
Nakamura, S.8
Nishimura, M.9
Akiguchi, I.10
Kimura, J.11
Narumiya, S.12
Kakizuka, A.13
-
34
-
-
0031012399
-
Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the α1A-voltage-dependent calcium channel
-
Zhuchenko, O.; Bailey, J.; Bonnen, P.; Ashizawa, T.; Stockton, D.W.; Amos, C.; Dobyns, W.B.; Subramony, S.H.; Zoghbi, H.Y.; Lee, C.C. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the α1A-voltage-dependent calcium channel. Nat. Genet., 1997, 15(1), 62-69.
-
(1997)
Nat. Genet
, vol.15
, Issue.1
, pp. 62-69
-
-
Zhuchenko, O.1
Bailey, J.2
Bonnen, P.3
Ashizawa, T.4
Stockton, D.W.5
Amos, C.6
Dobyns, W.B.7
Subramony, S.H.8
Zoghbi, H.Y.9
Lee, C.C.10
-
35
-
-
16944364511
-
Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion
-
David, G.; Abbas, N.; Stevanin, G.; Durr, A.; Yvert, G.; Cancel, G.; Weber, C.; Imbert, G.; Saudou, F.; Antoniou, E.; Drabkin, H.; Gemmill, R.; Giunti, P.; Benomar, A.; Wood, N.; Ruberg, M.; Agid, Y.; Mandel, J.L.; Brice, A. Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion. Nat. Genet., 1997, 17(1), 65-70.
-
(1997)
Nat. Genet
, vol.17
, Issue.1
, pp. 65-70
-
-
David, G.1
Abbas, N.2
Stevanin, G.3
Durr, A.4
Yvert, G.5
Cancel, G.6
Weber, C.7
Imbert, G.8
Saudou, F.9
Antoniou, E.10
Drabkin, H.11
Gemmill, R.12
Giunti, P.13
Benomar, A.14
Wood, N.15
Ruberg, M.16
Agid, Y.17
Mandel, J.L.18
Brice, A.19
-
36
-
-
0035393427
-
SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein
-
Nakamura, K.; Jeong, S.Y.; Uchihara, T.; Anno, M.; Nagashima, K.; Nagashima, T.; Ikeda, S.; Tsuji, S.; Kanazawa, I. SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum. Mol. Genet., 2001, 10(14), 1441-1448.
-
(2001)
Hum. Mol. Genet
, vol.10
, Issue.14
, pp. 1441-1448
-
-
Nakamura, K.1
Jeong, S.Y.2
Uchihara, T.3
Anno, M.4
Nagashima, K.5
Nagashima, T.6
Ikeda, S.7
Tsuji, S.8
Kanazawa, I.9
-
37
-
-
0028216760
-
Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)
-
Koide, R.; Ikeuchi, T.; Onodera, O.; Tanaka, H.; Igarashi, S.; Endo, K.; Takahashi, H.; Kondo, R.; Ishikawa, A.; Hayashi, T.; Saito, M.; Tomoda, A.; Miike, T.; Naito, H.; Ikuta, F.; Tsuji, S. Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nat. Genet., 1994, 6(1), 9-13.
-
(1994)
Nat. Genet
, vol.6
, Issue.1
, pp. 9-13
-
-
Koide, R.1
Ikeuchi, T.2
Onodera, O.3
Tanaka, H.4
Igarashi, S.5
Endo, K.6
Takahashi, H.7
Kondo, R.8
Ishikawa, A.9
Hayashi, T.10
Saito, M.11
Tomoda, A.12
Miike, T.13
Naito, H.14
Ikuta, F.15
Tsuji, S.16
-
38
-
-
0028335386
-
-
Nagafuchi, S.; Yanagisawa, H.; Sato, K.; Shirayama, T.; Ohsaki, E.; Bundo, M.; Takeda, T.; Tadokoro, K.; Kondo, I.; Murayama, N; Tanaka, Y.; Kikushima, H.; Umino, K.; Kurosawa, H.; Furukawa, T.; Nihei, K.; Inoue, T.; Sano, A.; Komure, O.; Takahashi, M.; Yoshizawa, T.; Kanazawa, I.; Yamada, M. Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p. Nat. Genet., 1994, 6(1), 14-18.
-
Nagafuchi, S.; Yanagisawa, H.; Sato, K.; Shirayama, T.; Ohsaki, E.; Bundo, M.; Takeda, T.; Tadokoro, K.; Kondo, I.; Murayama, N; Tanaka, Y.; Kikushima, H.; Umino, K.; Kurosawa, H.; Furukawa, T.; Nihei, K.; Inoue, T.; Sano, A.; Komure, O.; Takahashi, M.; Yoshizawa, T.; Kanazawa, I.; Yamada, M. Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p. Nat. Genet., 1994, 6(1), 14-18.
-
-
-
-
39
-
-
0025800526
-
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
-
La Spada, A.R.; Wilson, E.M.; Lubahn, D.B.; Harding, A.E.; Fischbeck, K.H. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature, 1991, 352(6330), 77-79.
-
(1991)
Nature
, vol.352
, Issue.6330
, pp. 77-79
-
-
La Spada, A.R.1
Wilson, E.M.2
Lubahn, D.B.3
Harding, A.E.4
Fischbeck, K.H.5
-
40
-
-
0029163222
-
SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat
-
Burright, E.N.; Clark, H.B.; Servadio, A.; Matilla, T.; Feddersen, R.M.; Yunis, W.S.; Duvick, L.A.; Zoghbi, H.Y.; Orr, H.T. SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell, 1995, 82(6), 937-948.
-
(1995)
Cell
, vol.82
, Issue.6
, pp. 937-948
-
-
Burright, E.N.1
Clark, H.B.2
Servadio, A.3
Matilla, T.4
Feddersen, R.M.5
Yunis, W.S.6
Duvick, L.A.7
Zoghbi, H.Y.8
Orr, H.T.9
-
41
-
-
0030058208
-
Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo
-
Ikeda, H.; Yamaguchi, M.; Sugai, S.; Aze, Y.; Narumiya, S.; Kakizuka, A. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nat. Genet., 1996, 13(2), 196-202.
-
(1996)
Nat. Genet
, vol.13
, Issue.2
, pp. 196-202
-
-
Ikeda, H.1
Yamaguchi, M.2
Sugai, S.3
Aze, Y.4
Narumiya, S.5
Kakizuka, A.6
-
42
-
-
16044373842
-
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
-
Mangiarini, L.; Sathasivam, K.; Seller, M.; Cozens, B.; Harper, A.; Hetherington, C.; Lawton, M.; Trottier, Y.; Lehrach, H.; Davies, S.W.; Bates, G.P. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell, 1996, 87(3), 493-506.
-
(1996)
Cell
, vol.87
, Issue.3
, pp. 493-506
-
-
Mangiarini, L.1
Sathasivam, K.2
Seller, M.3
Cozens, B.4
Harper, A.5
Hetherington, C.6
Lawton, M.7
Trottier, Y.8
Lehrach, H.9
Davies, S.W.10
Bates, G.P.11
-
43
-
-
18544392423
-
-
Warrick, J.M.; Paulson, H.L.; Gray-Board, G.L.; Bui, Q.T.; Fischbeck, K.H.; Pittman, R.N.; Bonini, N.M. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell, 1998, 93(6), 939-949.
-
Warrick, J.M.; Paulson, H.L.; Gray-Board, G.L.; Bui, Q.T.; Fischbeck, K.H.; Pittman, R.N.; Bonini, N.M. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell, 1998, 93(6), 939-949.
-
-
-
-
44
-
-
0033524413
-
Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron
-
Faber, P.W.; Alter, J.R.; MacDonald, M.E.; Hart, A.C. Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc. Natl. Acad. Sci. USA, 1999, 96(1), 179-184.
-
(1999)
Proc. Natl. Acad. Sci. USA
, vol.96
, Issue.1
, pp. 179-184
-
-
Faber, P.W.1
Alter, J.R.2
MacDonald, M.E.3
Hart, A.C.4
-
45
-
-
37849030901
-
Polyglutamine diseases: Emerging concepts in pathogenesis and therapy
-
Shao, J.; Diamond, M.I. Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum. Mol. Genet., 2007, 16(Spec No. 2), R115-123.
-
(2007)
Hum. Mol. Genet
, vol.16
, Issue.SPEC 2
-
-
Shao, J.1
Diamond, M.I.2
-
46
-
-
52049093169
-
Polyglutamine neurodegeneration: Protein misfolding revisited
-
Williams, A.J.; Paulson, H.L. Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci., 2008, 31(10), 521-528.
-
(2008)
Trends Neurosci
, vol.31
, Issue.10
, pp. 521-528
-
-
Williams, A.J.1
Paulson, H.L.2
-
47
-
-
3042717240
-
Cellular toxicity of polyglutamine expansion proteins: Mechanism of transcription factor deactivation
-
Schaffar, G.; Breuer, P.; Boteva, R.; Behrends, C.; Tzvetkov, N.; Strippel, N.; Sakahira, H.; Siegers, K.; Hayer-Hartl, M.; Hartl, F.U. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol. Cell, 2004, 15(1), 95-105.
-
(2004)
Mol. Cell
, vol.15
, Issue.1
, pp. 95-105
-
-
Schaffar, G.1
Breuer, P.2
Boteva, R.3
Behrends, C.4
Tzvetkov, N.5
Strippel, N.6
Sakahira, H.7
Siegers, K.8
Hayer-Hartl, M.9
Hartl, F.U.10
-
48
-
-
34047269594
-
Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy
-
Li, M.; Chevalier-Larsen, E.S.; Merry, D.E.; Diamond, M.I. Soluble androgen receptor oligomers underlie pathology in a mouse model of spinobulbar muscular atrophy. J. Biol. Chem., 2007, 282(5), 3157-3164.
-
(2007)
J. Biol. Chem
, vol.282
, Issue.5
, pp. 3157-3164
-
-
Li, M.1
Chevalier-Larsen, E.S.2
Merry, D.E.3
Diamond, M.I.4
-
49
-
-
34247247115
-
A toxic monomeric conformer of the polyglutamine protein
-
Nagai, Y.; Inui, T.; Popiel, H.A.; Fujikake, N.; Hasegawa, K.; Urade, Y.; Goto, Y.; Naiki, H.; Toda, T. A toxic monomeric conformer of the polyglutamine protein. Nat. Struct. Mol. Biol., 2007, 14(4), 332-340.
-
(2007)
Nat. Struct. Mol. Biol
, vol.14
, Issue.4
, pp. 332-340
-
-
Nagai, Y.1
Inui, T.2
Popiel, H.A.3
Fujikake, N.4
Hasegawa, K.5
Urade, Y.6
Goto, Y.7
Naiki, H.8
Toda, T.9
-
50
-
-
38349158062
-
Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic
-
Takahashi, T.; Kikuchi, S.; Katada, S.; Nagai, Y.; Nishizawa, M.; Onodera, O. Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum. Mol. Genet., 2008, 17(3), 345-356.
-
(2008)
Hum. Mol. Genet
, vol.17
, Issue.3
, pp. 345-356
-
-
Takahashi, T.1
Kikuchi, S.2
Katada, S.3
Nagai, Y.4
Nishizawa, M.5
Onodera, O.6
-
51
-
-
38449083555
-
The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias
-
Davies, J.E.; Sarkar, S.; Rubinsztein, D.C. The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias. BMC Biochem., 2007, 8(Suppl. 1), S2.
-
(2007)
BMC Biochem
, vol.8
, Issue.SUPPL. 1
-
-
Davies, J.E.1
Sarkar, S.2
Rubinsztein, D.C.3
-
52
-
-
53749088439
-
Intracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseases
-
Li, X.; Li, H.; Li, X.J. Intracellular degradation of misfolded proteins in polyglutamine neurodegenerative diseases. Brain Res. Rev., 2008, 59(1), 245-252.
-
(2008)
Brain Res. Rev
, vol.59
, Issue.1
, pp. 245-252
-
-
Li, X.1
Li, H.2
Li, X.J.3
-
53
-
-
0031838352
-
Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
-
Cummings, C.J.; Mancini, M.A.; Antalffy, B.; DeFranco, D.B.; Orr, H.T.; Zoghbi, H.Y. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet., 1998, 19(2), 148-154.
-
(1998)
Nat. Genet
, vol.19
, Issue.2
, pp. 148-154
-
-
Cummings, C.J.1
Mancini, M.A.2
Antalffy, B.3
DeFranco, D.B.4
Orr, H.T.5
Zoghbi, H.Y.6
-
54
-
-
0033499931
-
Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease
-
Chai, Y.; Koppenhafer, S.L.; Bonini, N.M.; Paulson, H.L. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J. Neurosci., 1999, 19(23), 10338-10347.
-
(1999)
J. Neurosci
, vol.19
, Issue.23
, pp. 10338-10347
-
-
Chai, Y.1
Koppenhafer, S.L.2
Bonini, N.M.3
Paulson, H.L.4
-
55
-
-
0032517816
-
Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation
-
Perez, M.K.; Paulson, H.L.; Pendse, S.J.; Saionz, S.J.; Bonini, N.M.; Pittman, R.N. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J. Cell. Biol., 1998, 143(6), 1457-1470.
-
(1998)
J. Cell. Biol
, vol.143
, Issue.6
, pp. 1457-1470
-
-
Perez, M.K.1
Paulson, H.L.2
Pendse, S.J.3
Saionz, S.J.4
Bonini, N.M.5
Pittman, R.N.6
-
56
-
-
0033818112
-
-
Shimohata, T.; Nakajima, T.; Yamada, M.; Uchida, C.; Onodera, O.; Naruse, S.; Kimura, T.; Koide, R.; Nozaki, K.; Sano, Y.; Ishiguro, H.; Sakoe, K.; Ooshima, T.; Sato, A.; Ikeuchi, T.; Oyake, M.; Sato, T.; Aoyagi, Y.; Hozumi, I.; Nagatsu, T.; Takiyama, Y.; Nishizawa, M.; Goto, J.; Kanazawa, I.; Davidson, I.; Tanese, N.; Takahashi, H.; Tsuji, S. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet., 2000, 26(1), 29-36.
-
Shimohata, T.; Nakajima, T.; Yamada, M.; Uchida, C.; Onodera, O.; Naruse, S.; Kimura, T.; Koide, R.; Nozaki, K.; Sano, Y.; Ishiguro, H.; Sakoe, K.; Ooshima, T.; Sato, A.; Ikeuchi, T.; Oyake, M.; Sato, T.; Aoyagi, Y.; Hozumi, I.; Nagatsu, T.; Takiyama, Y.; Nishizawa, M.; Goto, J.; Kanazawa, I.; Davidson, I.; Tanese, N.; Takahashi, H.; Tsuji, S. Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet., 2000, 26(1), 29-36.
-
-
-
-
57
-
-
0035937523
-
Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity
-
Nucifora, F.C., Jr.; Sasaki, M.; Peters, M.F.; Huang, H.; Cooper, J.K.; Yamada, M.; Takahashi, H.; Tsuji, S.; Troncoso, J.; Dawson, V.L.; Dawson, T.M.; Ross, C.A. Interference by huntingtin and atrophin-1 with CBP-mediated transcription leading to cellular toxicity. Science, 2001, 291(5512), 2423-2428.
-
(2001)
Science
, vol.291
, Issue.5512
, pp. 2423-2428
-
-
Nucifora Jr., F.C.1
Sasaki, M.2
Peters, M.F.3
Huang, H.4
Cooper, J.K.5
Yamada, M.6
Takahashi, H.7
Tsuji, S.8
Troncoso, J.9
Dawson, V.L.10
Dawson, T.M.11
Ross, C.A.12
-
58
-
-
0032976739
-
Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network
-
Nagai, Y.; Onodera, O.; Chun, J.; Strittmatter, W.J.; Burke, J.R. Expanded polyglutamine domain proteins bind neurofilament and alter the neurofilament network. Exp. Neurol., 1999, 155(2), 195-203.
-
(1999)
Exp. Neurol
, vol.155
, Issue.2
, pp. 195-203
-
-
Nagai, Y.1
Onodera, O.2
Chun, J.3
Strittmatter, W.J.4
Burke, J.R.5
-
59
-
-
0037154229
-
Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment
-
Muchowski, P.J.; Ning, K.; D'Souza-Schorey, C.; Fields, S. Requirement of an intact microtubule cytoskeleton for aggregation and inclusion body formation by a mutant huntingtin fragment. Proc. Natl. Acad. Sci. USA, 2002, 99(2), 727-732.
-
(2002)
Proc. Natl. Acad. Sci. USA
, vol.99
, Issue.2
, pp. 727-732
-
-
Muchowski, P.J.1
Ning, K.2
D'Souza-Schorey, C.3
Fields, S.4
-
60
-
-
20044377268
-
Early changes in Huntington's disease patient brains involve alterations in cytoskeletal and synaptic elements
-
DiProspero, N.A.; Chen, E.Y.; Charles, V.; Plomann, M.; Kordower, J.H.; Tagle, D.A. Early changes in Huntington's disease patient brains involve alterations in cytoskeletal and synaptic elements. J. Neurocytol., 2004, 33(5), 517-533.
-
(2004)
J. Neurocytol
, vol.33
, Issue.5
, pp. 517-533
-
-
DiProspero, N.A.1
Chen, E.Y.2
Charles, V.3
Plomann, M.4
Kordower, J.H.5
Tagle, D.A.6
-
61
-
-
0035947372
-
Impairment of the ubiquitin-proteasome system by protein aggregation
-
Bence, N.F.; Sampat, R.M.; Kopito, R.R. Impairment of the ubiquitin-proteasome system by protein aggregation. Science, 2001, 292(5521), 1552-1555.
-
(2001)
Science
, vol.292
, Issue.5521
, pp. 1552-1555
-
-
Bence, N.F.1
Sampat, R.M.2
Kopito, R.R.3
-
62
-
-
0035909330
-
Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
-
Steffan, J.S.; Bodai, L.; Pallos, J.; Poelman, M.; McCampbell, A.; Apostol, B.L.; Kazantsev, A.; Schmidt, E.; Zhu, Y.Z.; Greenwald, M.; Kurokawa, R.; Housman, D.E.; Jackson, G.R.; Marsh, J.L.; Thompson, L.M. Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature, 2001, 413(6857), 739-743.
-
(2001)
Nature
, vol.413
, Issue.6857
, pp. 739-743
-
-
Steffan, J.S.1
Bodai, L.2
Pallos, J.3
Poelman, M.4
McCampbell, A.5
Apostol, B.L.6
Kazantsev, A.7
Schmidt, E.8
Zhu, Y.Z.9
Greenwald, M.10
Kurokawa, R.11
Housman, D.E.12
Jackson, G.R.13
Marsh, J.L.14
Thompson, L.M.15
-
63
-
-
0141750470
-
Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila
-
Gunawardena, S.; Her, L.S.; Brusch, R.G.; Laymon, R.A.; Niesman, I.R.; Gordesky-Gold, B.; Sintasath, L.; Bonini, N.M.; Goldstein, L.S. Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. Neuron, 2003, 40(1), 25-40.
-
(2003)
Neuron
, vol.40
, Issue.1
, pp. 25-40
-
-
Gunawardena, S.1
Her, L.S.2
Brusch, R.G.3
Laymon, R.A.4
Niesman, I.R.5
Gordesky-Gold, B.6
Sintasath, L.7
Bonini, N.M.8
Goldstein, L.S.9
-
64
-
-
10744224530
-
Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport
-
Szebenyi, G.; Morfini, G.A.; Babcock, A.; Gould, M.; Selkoe, K.; Stenoien, D.L.; Young, M.; Faber, P.W.; MacDonald, M.E.; McPhaul, M.J.; Brady, S.T. Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport. Neuron, 2003, 40(1), 41-52.
-
(2003)
Neuron
, vol.40
, Issue.1
, pp. 41-52
-
-
Szebenyi, G.1
Morfini, G.A.2
Babcock, A.3
Gould, M.4
Selkoe, K.5
Stenoien, D.L.6
Young, M.7
Faber, P.W.8
MacDonald, M.E.9
McPhaul, M.J.10
Brady, S.T.11
-
65
-
-
25844506224
-
Therapeutics development for triplet repeat expansion diseases
-
Di Prospero, N.A.; Fischbeck, K.H. Therapeutics development for triplet repeat expansion diseases. Nat. Rev. Genet., 2005, 6(10), 756-765.
-
(2005)
Nat. Rev. Genet
, vol.6
, Issue.10
, pp. 756-765
-
-
Di Prospero, N.A.1
Fischbeck, K.H.2
-
66
-
-
44449131447
-
Huntington's disease: From pathology and genetics to potential therapies
-
Imarisio, S.; Carmichael, J.; Korolchuk, V.; Chen, C.W.; Saiki, S.; Rose, C.; Krishna, G.; Davies, J.E.; Ttofi, E.; Underwood, B.R.; Rubinsztein, D.C. Huntington's disease: from pathology and genetics to potential therapies. Biochem. J., 2008, 412(2), 191-209.
-
(2008)
Biochem. J
, vol.412
, Issue.2
, pp. 191-209
-
-
Imarisio, S.1
Carmichael, J.2
Korolchuk, V.3
Chen, C.W.4
Saiki, S.5
Rose, C.6
Krishna, G.7
Davies, J.E.8
Ttofi, E.9
Underwood, B.R.10
Rubinsztein, D.C.11
-
67
-
-
0038701684
-
Huntingtin aggregation and toxicity in Huntington's disease
-
Bates, G. Huntingtin aggregation and toxicity in Huntington's disease. Lancet, 2003, 361(9369), 1642-1644.
-
(2003)
Lancet
, vol.361
, Issue.9369
, pp. 1642-1644
-
-
Bates, G.1
-
68
-
-
0141891215
-
Pathogenesis of polyglutamine disorders: Aggregation revisited
-
Michalik, A.; Van Broeckhoven, C. Pathogenesis of polyglutamine disorders: aggregation revisited. Hum. Mol. Genet., 2003, 12(Spec No. 2), R173-186.
-
(2003)
Hum. Mol. Genet
, vol.12
, Issue.SPEC 2
-
-
Michalik, A.1
Van Broeckhoven, C.2
-
69
-
-
33745750191
-
Therapeutic approaches to polyglutamine diseases: Combating protein misfolding and aggregation
-
Herbst, M.; Wanker, E.E. Therapeutic approaches to polyglutamine diseases: combating protein misfolding and aggregation. Curr. Pharm. Des., 2006, 12(20), 2543-2555.
-
(2006)
Curr. Pharm. Des
, vol.12
, Issue.20
, pp. 2543-2555
-
-
Herbst, M.1
Wanker, E.E.2
-
70
-
-
0032945938
-
Polyglutamine- expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
-
Stenoien, D.L.; Cummings, C.J.; Adams, H.P.; Mancini, M.G.; Patel, K.; DeMartino, G.N.; Marcelli, M.; Weigel, N.L.; Mancini, M.A. Polyglutamine- expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet., 1999, 8(5), 731-741.
-
(1999)
Hum. Mol. Genet
, vol.8
, Issue.5
, pp. 731-741
-
-
Stenoien, D.L.1
Cummings, C.J.2
Adams, H.P.3
Mancini, M.G.4
Patel, K.5
DeMartino, G.N.6
Marcelli, M.7
Weigel, N.L.8
Mancini, M.A.9
-
71
-
-
0034708793
-
Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract
-
Kobayashi, Y.; Kume, A.; Li, M.; Doyu, M.; Hata, M.; Ohtsuka, K.; Sobue, G. Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor protein with expanded polyglutamine tract. J. Biol. Chem., 2000, 275(12), 8772-8778.
-
(2000)
J. Biol. Chem
, vol.275
, Issue.12
, pp. 8772-8778
-
-
Kobayashi, Y.1
Kume, A.2
Li, M.3
Doyu, M.4
Hata, M.5
Ohtsuka, K.6
Sobue, G.7
-
72
-
-
0034641589
-
Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: Their role in suppression of aggregation and cellular toxicity
-
Jana, N.R.; Tanaka, M.; Wang, G.; Nukina, N. Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity. Hum. Mol. Genet., 2000, 9(13), 2009-2018.
-
(2000)
Hum. Mol. Genet
, vol.9
, Issue.13
, pp. 2009-2018
-
-
Jana, N.R.1
Tanaka, M.2
Wang, G.3
Nukina, N.4
-
73
-
-
0032727617
-
-
Warrick, J.M.; Chan, H.Y.; Gray-Board, G.L.; Chai, Y.; Paulson, H.L.; Bonini, N.M. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet., 1999, 23(4), 425-428.
-
Warrick, J.M.; Chan, H.Y.; Gray-Board, G.L.; Chai, Y.; Paulson, H.L.; Bonini, N.M. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet., 1999, 23(4), 425-428.
-
-
-
-
74
-
-
0034629073
-
Genetic suppression of polyglutamine toxicity in Drosophila
-
Kazemi-Esfarjani, P.; Benzer, S. Genetic suppression of polyglutamine toxicity in Drosophila. Science, 2000, 287(5459), 1837-1840.
-
(2000)
Science
, vol.287
, Issue.5459
, pp. 1837-1840
-
-
Kazemi-Esfarjani, P.1
Benzer, S.2
-
75
-
-
0034597833
-
Identification of genes that modify ataxin-1-induced neurodegeneration
-
Fernandez-Funez, P.; Nino-Rosales, M.L.; de Gouyon, B.; She, W.C.; Luchak, J.M.; Martinez, P.; Turiegano, E.; Benito, J.; Capovilla, M.; Skinner, P.J.; McCall, A.; Canal, I.; Orr, H.T.; Zoghbi, H.Y.; Botas, J. Identification of genes that modify ataxin-1-induced neurodegeneration. Nature, 2000, 408(6808), 101-106.
-
(2000)
Nature
, vol.408
, Issue.6808
, pp. 101-106
-
-
Fernandez-Funez, P.1
Nino-Rosales, M.L.2
de Gouyon, B.3
She, W.C.4
Luchak, J.M.5
Martinez, P.6
Turiegano, E.7
Benito, J.8
Capovilla, M.9
Skinner, P.J.10
McCall, A.11
Canal, I.12
Orr, H.T.13
Zoghbi, H.Y.14
Botas, J.15
-
76
-
-
0034703863
-
-
Chan, H.Y.; Warrick, J.M.; Gray-Board, G.L.; Paulson, H.L.; Bonini, N.M. Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Hum. Mol. Genet., 2000, 9(19), 2811-2820.
-
Chan, H.Y.; Warrick, J.M.; Gray-Board, G.L.; Paulson, H.L.; Bonini, N.M. Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Hum. Mol. Genet., 2000, 9(19), 2811-2820.
-
-
-
-
77
-
-
0034608868
-
Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils
-
Muchowski, P.J.; Schaffar, G.; Sittler, A.; Wanker, E.E.; Hayer-Hartl, M.K.; Hartl, F.U. Hsp70 and Hsp40 chaperones can inhibit self-assembly of polyglutamine proteins into amyloid-like fibrils. Proc. Natl. Acad. Sci. USA, 2000, 97(14), 7841-7846.
-
(2000)
Proc. Natl. Acad. Sci. USA
, vol.97
, Issue.14
, pp. 7841-7846
-
-
Muchowski, P.J.1
Schaffar, G.2
Sittler, A.3
Wanker, E.E.4
Hayer-Hartl, M.K.5
Hartl, F.U.6
-
78
-
-
0035394668
-
Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice
-
Cummings, C.J.; Sun, Y.; Opal, P.; Antalffy, B.; Mestril, R.; Orr, H.T.; Dillmann, W.H.; Zoghbi, H.Y. Over-expression of inducible HSP70 chaperone suppresses neuropathology and improves motor function in SCA1 mice. Hum. Mol. Genet., 2001, 10(14), 1511-1518.
-
(2001)
Hum. Mol. Genet
, vol.10
, Issue.14
, pp. 1511-1518
-
-
Cummings, C.J.1
Sun, Y.2
Opal, P.3
Antalffy, B.4
Mestril, R.5
Orr, H.T.6
Dillmann, W.H.7
Zoghbi, H.Y.8
-
79
-
-
0037444446
-
Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein
-
Adachi, H.; Katsuno, M.; Minamiyama, M.; Sang, C.; Pagoulatos, G.; Angelidis, C.; Kusakabe, M.; Yoshiki, A.; Kobayashi, Y.; Doyu, M.; Sobue, G. Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J. Neurosci., 2003, 23(6), 2203-2211.
-
(2003)
J. Neurosci
, vol.23
, Issue.6
, pp. 2203-2211
-
-
Adachi, H.1
Katsuno, M.2
Minamiyama, M.3
Sang, C.4
Pagoulatos, G.5
Angelidis, C.6
Kusakabe, M.7
Yoshiki, A.8
Kobayashi, Y.9
Doyu, M.10
Sobue, G.11
-
80
-
-
0347928859
-
Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression
-
Hansson, O.; Nylandsted, J.; Castilho, R.F.; Leist, M.; Jaattela, M.; Brundin, P. Overexpression of heat shock protein 70 in R6/2 Huntington's disease mice has only modest effects on disease progression. Brain Res., 2003, 970(1-2), 47-57.
-
(2003)
Brain Res
, vol.970
, Issue.1-2
, pp. 47-57
-
-
Hansson, O.1
Nylandsted, J.2
Castilho, R.F.3
Leist, M.4
Jaattela, M.5
Brundin, P.6
-
81
-
-
3242695184
-
Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
-
Hay, D.G.; Sathasivam, K.; Tobaben, S.; Stahl, B.; Marber, M.; Mestril, R.; Mahal, A.; Smith, D.L.; Woodman, B.; Bates, G.P. Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach. Hum. Mol. Genet., 2004, 13(13), 1389-1405.
-
(2004)
Hum. Mol. Genet
, vol.13
, Issue.13
, pp. 1389-1405
-
-
Hay, D.G.1
Sathasivam, K.2
Tobaben, S.3
Stahl, B.4
Marber, M.5
Mestril, R.6
Mahal, A.7
Smith, D.L.8
Woodman, B.9
Bates, G.P.10
-
82
-
-
11244281642
-
Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice
-
Helmlinger, D.; Bonnet, J.; Mandel, J.L.; Trottier, Y.; Devys, D. Hsp70 and Hsp40 chaperones do not modulate retinal phenotype in SCA7 mice. J. Biol. Chem., 2004, 279(53), 55969-55977.
-
(2004)
J. Biol. Chem
, vol.279
, Issue.53
, pp. 55969-55977
-
-
Helmlinger, D.1
Bonnet, J.2
Mandel, J.L.3
Trottier, Y.4
Devys, D.5
-
83
-
-
67650745109
-
Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease
-
Wacker, J.L.; Huang, S.Y.; Steele, A.D.; Aron, R.; Lotz, G.P.; Nguyen, Q.; Giorgini, F.; Roberson, E.D.; Lindquist, S.; Masliah, E.; Muchowski, P.J. Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease. J. Neurosci., 2009, 29(28), 9104-9114.
-
(2009)
J. Neurosci
, vol.29
, Issue.28
, pp. 9104-9114
-
-
Wacker, J.L.1
Huang, S.Y.2
Steele, A.D.3
Aron, R.4
Lotz, G.P.5
Nguyen, Q.6
Giorgini, F.7
Roberson, E.D.8
Lindquist, S.9
Masliah, E.10
Muchowski, P.J.11
-
84
-
-
0036566675
-
Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin
-
Wyttenbach, A.; Sauvageot, O.; Carmichael, J.; Diaz-Latoud, C.; Arrigo, A.P.; Rubinsztein, D.C. Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin. Hum. Mol. Genet., 2002, 11(9), 1137-1151.
-
(2002)
Hum. Mol. Genet
, vol.11
, Issue.9
, pp. 1137-1151
-
-
Wyttenbach, A.1
Sauvageot, O.2
Carmichael, J.3
Diaz-Latoud, C.4
Arrigo, A.P.5
Rubinsztein, D.C.6
-
85
-
-
21244489544
-
HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells
-
Carra, S.; Sivilotti, M.; Chavez Z.A.T.; Lambert, H.; Landry, J. HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells. Hum. Mol. Genet., 2005, 14(12), 1659-1669.
-
(2005)
Hum. Mol. Genet
, vol.14
, Issue.12
, pp. 1659-1669
-
-
Carra, S.1
Sivilotti, M.2
Chavez, Z.A.T.3
Lambert, H.4
Landry, J.5
-
86
-
-
0036848793
-
Purification of polyglutamine aggregates and identification of elongation factor-1α and heat shock protein 84 as aggregate-interacting proteins
-
Mitsui, K.; Nakayama, H.; Akagi, T.; Nekooki, M.; Ohtawa, K.; Takio, K.; Hashikawa, T.; Nukina, N. Purification of polyglutamine aggregates and identification of elongation factor-1α and heat shock protein 84 as aggregate-interacting proteins. J. Neurosci., 2002, 22(21), 9267-9277.
-
(2002)
J. Neurosci
, vol.22
, Issue.21
, pp. 9267-9277
-
-
Mitsui, K.1
Nakayama, H.2
Akagi, T.3
Nekooki, M.4
Ohtawa, K.5
Takio, K.6
Hashikawa, T.7
Nukina, N.8
-
87
-
-
0042591262
-
Hsp105α suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity
-
Ishihara, K.; Yamagishi, N.; Saito, Y.; Adachi, H.; Kobayashi, Y.; Sobue, G.; Ohtsuka, K.; Hatayama, T. Hsp105α suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. J. Biol. Chem., 2003, 278(27), 25143-25150.
-
(2003)
J. Biol. Chem
, vol.278
, Issue.27
, pp. 25143-25150
-
-
Ishihara, K.1
Yamagishi, N.2
Saito, Y.3
Adachi, H.4
Kobayashi, Y.5
Sobue, G.6
Ohtsuka, K.7
Hatayama, T.8
-
88
-
-
33748561495
-
Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers
-
Behrends, C.; Langer, C.A.; Boteva, R.; Bottcher, U.M.; Stemp, M.J.; Schaffar, G.; Rao, B.V.; Giese, A.; Kretzschmar, H.; Siegers, K.; Hartl, F.U. Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. Mol. Cell, 2006, 23(6), 887-897.
-
(2006)
Mol. Cell
, vol.23
, Issue.6
, pp. 887-897
-
-
Behrends, C.1
Langer, C.A.2
Boteva, R.3
Bottcher, U.M.4
Stemp, M.J.5
Schaffar, G.6
Rao, B.V.7
Giese, A.8
Kretzschmar, H.9
Siegers, K.10
Hartl, F.U.11
-
89
-
-
33749176269
-
Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state
-
Kitamura, A.; Kubota, H.; Pack, C.G.; Matsumoto, G.; Hirayama, S.; Takahashi, Y.; Kimura, H.; Kinjo, M.; Morimoto, R.I.; Nagata, K. Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. Nat. Cell Biol., 2006, 8(10), 1163-1170.
-
(2006)
Nat. Cell Biol
, vol.8
, Issue.10
, pp. 1163-1170
-
-
Kitamura, A.1
Kubota, H.2
Pack, C.G.3
Matsumoto, G.4
Hirayama, S.5
Takahashi, Y.6
Kimura, H.7
Kinjo, M.8
Morimoto, R.I.9
Nagata, K.10
-
90
-
-
33749177252
-
The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions
-
Tam, S.; Geller, R.; Spiess, C.; Frydman, J. The chaperonin TRiC controls polyglutamine aggregation and toxicity through subunit-specific interactions. Nat. Cell Biol., 2006, 8(10), 1155-1162.
-
(2006)
Nat. Cell Biol
, vol.8
, Issue.10
, pp. 1155-1162
-
-
Tam, S.1
Geller, R.2
Spiess, C.3
Frydman, J.4
-
91
-
-
15244363187
-
BAG-1 associates with the polyglutamine-expanded huntingtin aggregates
-
Jana, N.R.; Nukina, N. BAG-1 associates with the polyglutamine-expanded huntingtin aggregates. Neurosci. Lett., 2005, 378(3), 171-175.
-
(2005)
Neurosci. Lett
, vol.378
, Issue.3
, pp. 171-175
-
-
Jana, N.R.1
Nukina, N.2
-
92
-
-
15744387323
-
Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes
-
Jana, N.R.; Dikshit, P.; Goswami, A.; Kotliarova, S.; Murata, S.; Tanaka, K.; Nukina, N. Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. J. Biol. Chem., 2005, 280(12), 11635-11640.
-
(2005)
J. Biol. Chem
, vol.280
, Issue.12
, pp. 11635-11640
-
-
Jana, N.R.1
Dikshit, P.2
Goswami, A.3
Kotliarova, S.4
Murata, S.5
Tanaka, K.6
Nukina, N.7
-
93
-
-
27144558034
-
CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo
-
Miller, V.M.; Nelson, R.F.; Gouvion, C.M.; Williams, A.; Rodriguez-Lebron, E.; Harper, S.Q.; Davidson, B.L.; Rebagliati, M.R.; Paulson, H.L. CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J. Neurosci., 2005, 25(40), 9152-9161.
-
(2005)
J. Neurosci
, vol.25
, Issue.40
, pp. 9152-9161
-
-
Miller, V.M.1
Nelson, R.F.2
Gouvion, C.M.3
Williams, A.4
Rodriguez-Lebron, E.5
Harper, S.Q.6
Davidson, B.L.7
Rebagliati, M.R.8
Paulson, H.L.9
-
94
-
-
11144243412
-
Modulation of neurodegeneration by molecular chaperones
-
Muchowski, P.J.; Wacker, J.L. Modulation of neurodegeneration by molecular chaperones. Nat. Rev. Neurosci., 2005, 6(1), 11-22.
-
(2005)
Nat. Rev. Neurosci
, vol.6
, Issue.1
, pp. 11-22
-
-
Muchowski, P.J.1
Wacker, J.L.2
-
95
-
-
68649113747
-
The role of molecular chaperones in human misfolding diseases
-
Broadley, S.A.; Hartl, F.U. The role of molecular chaperones in human misfolding diseases. FEBS Lett., 2009, 583(16), 2647-2653.
-
(2009)
FEBS Lett
, vol.583
, Issue.16
, pp. 2647-2653
-
-
Broadley, S.A.1
Hartl, F.U.2
-
96
-
-
0036797242
-
Polyglutamine protein aggregates are dynamic
-
Kim, S.; Nollen, E.A.; Kitagawa, K.; Bindokas, V.P.; Morimoto, R.I. Polyglutamine protein aggregates are dynamic. Nat. Cell Biol., 2002, 4(10), 826-831.
-
(2002)
Nat. Cell Biol
, vol.4
, Issue.10
, pp. 826-831
-
-
Kim, S.1
Nollen, E.A.2
Kitagawa, K.3
Bindokas, V.P.4
Morimoto, R.I.5
-
97
-
-
40949135766
-
Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor
-
Yamanaka, T.; Miyazaki, H.; Oyama, F.; Kurosawa, M.; Washizu, C.; Doi, H.; Nukina, N. Mutant Huntingtin reduces HSP70 expression through the sequestration of NF-Y transcription factor. EMBO J., 2008, 27(6), 827-839.
-
(2008)
EMBO J
, vol.27
, Issue.6
, pp. 827-839
-
-
Yamanaka, T.1
Miyazaki, H.2
Oyama, F.3
Kurosawa, M.4
Washizu, C.5
Doi, H.6
Nukina, N.7
-
98
-
-
23044487281
-
Dynamic regulation of molecular chaperone gene expression in polyglutamine disease
-
Huen, N.Y.; Chan, H.Y. Dynamic regulation of molecular chaperone gene expression in polyglutamine disease. Biochem. Biophys. Res. Commun., 2005, 334(4), 1074-1084.
-
(2005)
Biochem. Biophys. Res. Commun
, vol.334
, Issue.4
, pp. 1074-1084
-
-
Huen, N.Y.1
Chan, H.Y.2
-
99
-
-
33846611068
-
The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes
-
Tagawa, K.; Marubuchi, S.; Qi, M.L.; Enokido, Y.; Tamura, T.; Inagaki, R.; Murata, M.; Kanazawa, I.; Wanker, E.E.; Okazawa, H. The induction levels of heat shock protein 70 differentiate the vulnerabilities to mutant huntingtin among neuronal subtypes. J. Neurosci., 2007, 27(4), 868-880.
-
(2007)
J. Neurosci
, vol.27
, Issue.4
, pp. 868-880
-
-
Tagawa, K.1
Marubuchi, S.2
Qi, M.L.3
Enokido, Y.4
Tamura, T.5
Inagaki, R.6
Murata, M.7
Kanazawa, I.8
Wanker, E.E.9
Okazawa, H.10
-
100
-
-
0032476596
-
Xenopus NF-Y pre-sets chromatin to potentiate p300 and acetylation-responsive transcription from the Xenopus hsp70 promoter in vivo
-
Li, Q.; Herrler, M.; Landsberger, N.; Kaludov, N.; Ogryzko, V.V.; Nakatani, Y.; Wolffe, A.P. Xenopus NF-Y pre-sets chromatin to potentiate p300 and acetylation-responsive transcription from the Xenopus hsp70 promoter in vivo. EMBO J., 1998, 17(21), 6300-6315.
-
(1998)
EMBO J
, vol.17
, Issue.21
, pp. 6300-6315
-
-
Li, Q.1
Herrler, M.2
Landsberger, N.3
Kaludov, N.4
Ogryzko, V.V.5
Nakatani, Y.6
Wolffe, A.P.7
-
101
-
-
17744372861
-
Roles of the heat shock transcription factors in regulation of the heat shock response and beyond
-
Pirkkala, L.; Nykanen, P.; Sistonen, L. Roles of the heat shock transcription factors in regulation of the heat shock response and beyond. FASEB J., 2001, 15(7), 1118-1131.
-
(2001)
FASEB J
, vol.15
, Issue.7
, pp. 1118-1131
-
-
Pirkkala, L.1
Nykanen, P.2
Sistonen, L.3
-
102
-
-
44849094781
-
Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging
-
Morimoto, R.I. Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging. Genes Dev., 2008, 22(11), 1427-1438.
-
(2008)
Genes Dev
, vol.22
, Issue.11
, pp. 1427-1438
-
-
Morimoto, R.I.1
-
103
-
-
0035503470
-
Analysis of heat shock transcription factor for suppression of polyglutamine toxicity
-
Rimoldi, M.; Servadio, A.; Zimarino, V. Analysis of heat shock transcription factor for suppression of polyglutamine toxicity. Brain Res. Bull., 2001, 56(3-4), 353-362.
-
(2001)
Brain Res. Bull
, vol.56
, Issue.3-4
, pp. 353-362
-
-
Rimoldi, M.1
Servadio, A.2
Zimarino, V.3
-
104
-
-
27144524290
-
Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models
-
Fujimoto, M.; Takaki, E.; Hayashi, T.; Kitaura, Y.; Tanaka, Y.; Inouye, S.; Nakai, A. Active HSF1 significantly suppresses polyglutamine aggregate formation in cellular and mouse models. J. Biol. Chem., 2005, 280(41), 34908-34916.
-
(2005)
J. Biol. Chem
, vol.280
, Issue.41
, pp. 34908-34916
-
-
Fujimoto, M.1
Takaki, E.2
Hayashi, T.3
Kitaura, Y.4
Tanaka, Y.5
Inouye, S.6
Nakai, A.7
-
105
-
-
71949086967
-
Molecular pathogenesis of protein misfolding diseases: Pathological molecular environments versus quality control systems against misfolded proteins
-
Naiki, H.; Nagai, Y. Molecular pathogenesis of protein misfolding diseases: pathological molecular environments versus quality control systems against misfolded proteins. J. Biochem., 2009, 146(6), 751-756.
-
(2009)
J. Biochem
, vol.146
, Issue.6
, pp. 751-756
-
-
Naiki, H.1
Nagai, Y.2
-
106
-
-
0035363805
-
Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease
-
Sittler, A.; Lurz, R.; Lueder, G.; Priller, J.; Lehrach, H.; Hayer-Hartl, M.K.; Hartl, F.U.; Wanker, E.E. Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Hum. Mol. Genet., 2001, 10(12), 1307-1315.
-
(2001)
Hum. Mol. Genet
, vol.10
, Issue.12
, pp. 1307-1315
-
-
Sittler, A.1
Lurz, R.2
Lueder, G.3
Priller, J.4
Lehrach, H.5
Hayer-Hartl, M.K.6
Hartl, F.U.7
Wanker, E.E.8
-
107
-
-
28044469532
-
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease
-
Katsuno, M.; Sang, C.; Adachi, H.; Minamiyama, M.; Waza, M.; Tanaka, F.; Doyu, M.; Sobue, G. Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease. Proc. Natl. Acad. Sci. USA, 2005, 102(46), 16801-16806.
-
(2005)
Proc. Natl. Acad. Sci. USA
, vol.102
, Issue.46
, pp. 16801-16806
-
-
Katsuno, M.1
Sang, C.2
Adachi, H.3
Minamiyama, M.4
Waza, M.5
Tanaka, F.6
Doyu, M.7
Sobue, G.8
-
108
-
-
33845755480
-
The induction mechanism of the molecular chaperone HSP70 in the gastric mucosa by Geranylgeranylacetone (HSP-inducer)
-
Otaka, M.; Yamamoto, S.; Ogasawara, K.; Takaoka, Y.; Noguchi, S.; Miyazaki, T.; Nakai, A.; Odashima, M.; Matsuhashi, T.; Watanabe, S.; Itoh, H. The induction mechanism of the molecular chaperone HSP70 in the gastric mucosa by Geranylgeranylacetone (HSP-inducer). Biochem. Biophys. Res. Commun., 2007, 353(2), 399-404.
-
(2007)
Biochem. Biophys. Res. Commun
, vol.353
, Issue.2
, pp. 399-404
-
-
Otaka, M.1
Yamamoto, S.2
Ogasawara, K.3
Takaoka, Y.4
Noguchi, S.5
Miyazaki, T.6
Nakai, A.7
Odashima, M.8
Matsuhashi, T.9
Watanabe, S.10
Itoh, H.11
-
109
-
-
34347258879
-
Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy
-
Herbst, M.; Wanker, E.E. Small molecule inducers of heat-shock response reduce polyQ-mediated huntingtin aggregation. A possible therapeutic strategy. Neurodegener. Dis., 2007, 4(2-3), 254-260.
-
(2007)
Neurodegener. Dis
, vol.4
, Issue.2-3
, pp. 254-260
-
-
Herbst, M.1
Wanker, E.E.2
-
110
-
-
36849044616
-
Celastrol inhibits polyglutamine aggregation and toxicity though induction of the heat shock response
-
Zhang, Y.Q.; Sarge, K.D. Celastrol inhibits polyglutamine aggregation and toxicity though induction of the heat shock response. J. Mol. Med., 2007, 85(12), 1421-1428.
-
(2007)
J. Mol. Med
, vol.85
, Issue.12
, pp. 1421-1428
-
-
Zhang, Y.Q.1
Sarge, K.D.2
-
111
-
-
54449101793
-
Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones
-
Fujikake, N.; Nagai, Y.; Popiel, H.A.; Okamoto, Y.; Yamaguchi, M.; Toda, T. Heat shock transcription factor 1-activating compounds suppress polyglutamine-induced neurodegeneration through induction of multiple molecular chaperones. J. Biol. Chem., 2008, 283(38), 26188-26197.
-
(2008)
J. Biol. Chem
, vol.283
, Issue.38
, pp. 26188-26197
-
-
Fujikake, N.1
Nagai, Y.2
Popiel, H.A.3
Okamoto, Y.4
Yamaguchi, M.5
Toda, T.6
-
112
-
-
0036852712
-
Pharmacological prevention of Parkinson disease in Drosophila
-
Auluck, P.K.; Bonini, N.M. Pharmacological prevention of Parkinson disease in Drosophila. Nat. Med., 2002, 8(11), 1185-1186.
-
(2002)
Nat. Med
, vol.8
, Issue.11
, pp. 1185-1186
-
-
Auluck, P.K.1
Bonini, N.M.2
-
113
-
-
1942486792
-
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice
-
Kieran, D.; Kalmar, B.; Dick, J.R.; Riddoch-Contreras, J.; Burnstock, G.; Greensmith, L. Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice. Nat. Med., 2004, 10(4), 402-405.
-
(2004)
Nat. Med
, vol.10
, Issue.4
, pp. 402-405
-
-
Kieran, D.1
Kalmar, B.2
Dick, J.R.3
Riddoch-Contreras, J.4
Burnstock, G.5
Greensmith, L.6
-
114
-
-
33747691089
-
A phase II trial of 17-(Allylamino)-17- demethoxygeldanamycin in patients with papillary and clear cell renal cell carcinoma
-
Ronnen, E.A.; Kondagunta, G.V.; Ishill, N.; Sweeney, S.M.; Deluca, J.K.; Schwartz, L.; Bacik, J.; Motzer, R.J. A phase II trial of 17-(Allylamino)-17- demethoxygeldanamycin in patients with papillary and clear cell renal cell carcinoma. Invest. New Drugs, 2006, 24(6), 543-546.
-
(2006)
Invest. New Drugs
, vol.24
, Issue.6
, pp. 543-546
-
-
Ronnen, E.A.1
Kondagunta, G.V.2
Ishill, N.3
Sweeney, S.M.4
Deluca, J.K.5
Schwartz, L.6
Bacik, J.7
Motzer, R.J.8
|